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Congenital renal aplasia. What is renal agenesis and how to treat such a pathology? Agenesis of the left kidney

Bilateral anomaly ( complete absence kidney) – bilateral agenesis or arena. As a rule, the fetus dies in utero, or the born child dies in the first hours or days of life due to renal failure. Modern methods make it possible to combat this pathology with the help of organ transplantation and regular hemodialysis.
Agenesis of the right kidney - unilateral agenesis. This is an anatomical defect that is also congenital. Functional load A healthy kidney takes over, compensating for the deficiency as much as its structure and size allow.
Agenesis of the left kidney is an identical case of agenesis of the right kidney.
Aplasia of the right kidney is practically indistinguishable from agenesis, but the kidney is a rudimentary fibrous tissue without renal glomeruli, ureter and pelvis.
Aplasia of the left kidney is an anomaly identical to the underdevelopment of the right kidney.

Variants of agenesis are also possible, in which the ureter is preserved and functions quite normally; in the absence of the ureter, the clinical manifestations of the pathology are more pronounced.

As a rule, in clinical practice a unilateral anomaly occurs for obvious reasons - bilateral agenesis is not compatible with life.

Agenesis of the right kidney

By clinical manifestations agenesis of the right kidney is not much different from the anomaly of the left kidney, however, there is an opinion of authoritative urologists and nephrologists that the absence of the right kidney is much more common than agenesis of the left kidney, and in females. Perhaps this is due to anatomical specifics, because right kidney slightly smaller, shorter and more mobile than the left one, normally it should be located lower, which makes it more vulnerable. Agenesis of the right kidney can appear from the first days of the child’s birth if left kidney incapable of compensatory function. Symptoms of agenesis are polyuria (excessive urination), constant regurgitation, which can be classified as vomiting, total dehydration, hypertension, general intoxication and renal failure.

If the left kidney takes on the function of the missing right kidney, then agenesis of the right kidney practically does not manifest itself symptomatically and is discovered randomly. The diagnosis can be confirmed using computed tomography, ultrasound and urography. Also, the pediatrician, as well as parents, should be wary of excessive puffiness of the child’s face, a flattened wide nose (flat bridge of the nose and wide bridge of the nose), strongly protruding frontal lobes, too low located ears, possibly deformed. Ocular hypertelorism is not specific symptom, which indicates kidney agenesis, but often accompanies it as well as an enlarged abdomen and deformed lower limbs.

If agenesis of the right kidney does not pose a threat to health and does not manifest itself with obvious pathological symptoms, as a rule, this pathology does not require special treatment, the patient is under constant supervision of a urologist and undergoes regular screening examinations. It would be helpful to follow an adequate diet and preventive measures to reduce the risk of developing kidney diseases. If agenesis of the right kidney is accompanied by persistent renal hypertension or reverse reflux of urine from the ureters into the kidney, lifelong imprisonment is prescribed antihypertensive therapy, possible indications for organ transplantation.

Agenesis of the left kidney

This anomaly is almost identical to agenesis of the right kidney, except that normally the left kidney should be slightly more advanced than the right. Agenesis of the left kidney is a more complicated case, since its function should be performed by the right kidney, which is more mobile and less functional by nature. In addition, there is information, however, not confirmed by global urological statistics, that agenesis of the left kidney is more often accompanied by the absence of the ureteric orifice, this applies primarily to male patients. Similar pathology combined with agenesis of the seminal duct, underdevelopment bladder and anomaly of the seminal vesicles.

Visually pronounced agenesis of the left kidney can be determined by the same parameters as the agenesis of the right kidney, which are formed as a result of congenital intrauterine defects - oligohydramnios and fetal compression: a wide bridge of the nose, excessively widely spaced eyes (hypertelorism), a typical face with Potter syndrome - a puffy face with underdeveloped chin, low-set ears, with prominent epicanthic folds.

Agenesis of the left kidney in men is more pronounced in terms of symptoms; it manifests itself in constant pain V groin area, pain in the sacrum, difficulties with ejaculation, often leads to sexual dysfunction, impotence and infertility. The treatment required for agenesis of the left kidney depends on the degree of activity of the healthy right kidney. If the right kidney enlarges compensatoryly and functions normally, then it is only possible symptomatic treatment, including preventive antibacterial measures to reduce the risk of developing pyelonephritis or uropathology urinary system. A medical check-up with a nephrologist, and regular examinations of urine, blood, and ultrasound screening are also required. More severe cases of agenesis are considered an indication for kidney transplantation.

Aplasia of the right kidney

As a rule, underdevelopment of one of the kidneys is considered relatively favorable anomaly compared to agenesis. Aplasia of the right kidney may not appear during normal functioning of the healthy left kidney clinical signs all my life. Often, aplasia of the right kidney is diagnosed randomly when comprehensive examination about a completely different disease. Less commonly, it is identified as a possible cause of persistent hypertension or nephropathology. Only one third of all patients with an underdeveloped or “wrinkled” kidney, as it is also called, are registered with a nephrologist for aplasia during their lifetime. Clinical symptoms are nonspecific and, perhaps, this explains such a rare detection of this anomaly.

Among the signs that may indirectly indicate that a person may have underdeveloped one of the kidneys are periodic complaints of nagging pain in the lower abdomen, in the lumbar region. Painful sensations are associated with the growth of rudimentary fibrous tissue and pinched nerve endings. Also, one of the signs may be persistent hypertension that cannot be controlled adequate therapy. Aplasia of the right kidney, as a rule, does not require treatment. A gentle diet is needed to reduce the risk of strain on the hypertrophied kidney, performing a double function. Also, for persistent hypertension, appropriate treatment is prescribed with gentle diuretics. Aplasia of the right kidney has a favorable prognosis; usually people with one kidney live a full, high-quality life.

Aplasia of the left kidney

Aplasia of the left kidney, as well as aplasia of the right kidney, is quite rare, not more than 5-7% of all patients with anomalies of the urinary system. Aplasia is often combined with underdevelopment of nearby organs, for example, with an anomaly of the bladder. It is believed that aplasia of the left kidney is most often diagnosed in males and is accompanied by underdevelopment of the lungs and genital organs. In men, aplasia of the left kidney is diagnosed along with aplasia of the prostate, testicle and vas deferens. In women - underdevelopment of the uterine appendages, ureter, aplasia of the uterus itself (bicornuate uterus), aplasia intrauterine septum, vaginal doubling and so on.

An underdeveloped kidney does not have a leg or pelvis and is not able to function and produce urine. Aplasia of the left kidney, just like aplasia of the right kidney, is called in urological practice a solitary kidney, that is, a single kidney. This refers to only the kidney that is forced to function, compensatory to do double work.

Aplasia of the left kidney is detected randomly, since it does not manifest itself with clinically significant symptoms. Only functional changes And painful sensations in the collateral kidney may give rise to a urological examination.

The right kidney, which is forced to perform the work of the aplastic left kidney, is usually hypertrophied and may have cysts, but most often it has a completely normal structure and completely controls homeostasis.

Aplasia of the left kidney in both children and adults does not require specific treatment, with the exception of preventive measures to reduce risk bacterial infection in a single solitary kidney. Gentle diet, maintenance immune system, maximum avoidance of contracting viruses and infections ensures a completely healthy, full life a patient with one functioning kidney.

July 19, 2017 Doctor

Congenital malformations of the urinary system include renal agenesis. The term implies a complete absence paired organ, the remaining kidney begins to hypertrophy in order to take over functional responsibilities. The cause of the pathology is an exogenous effect on the embryo during embryonic development.

Characteristics of the anomaly

Renal agenesis refers to the absence of one or two organs. Pathology belongs to the category of anatomical quantitative anomalies, when part of the child’s urinary system has not formed during the prenatal period.

Kidney aplasia is an anomaly that occurs with similar symptoms and having similar reasons. In the case of aplasia, the organ is presented as a rudiment in the form of a connective tissue cord, which does not have a full-fledged stalk and pelvis. Due to the lack of natural kidney structure, it is unable to function normally. In both agenesis and aplasia, the second organ compensatory takes on all the work. Due to the increased daily load, its tissues hypertrophy, the kidney increases in size.

The cause of agenesis does not involve hereditary factor, only developmental anomalies during intrauterine maturation. In any case, the conditions for the appearance of this type of defect can be the following factors that are activated during pregnancy:

diseases caused by infection initial stage embryo development;
diagnostic examinations based on radiation;
abuse of a pregnant woman alcoholic drinks;
sexually transmitted diseases;
drugs with high content hormones.

If the expectant mother has a disease such as diabetes mellitus, the risk of pathology in a child increases significantly, especially close monitoring of the development of the fetus inside the womb is carried out.

Forms of development of the defect

Bilateral agenesis in medical field It is less common than unilateral, since the absence of both organs of the urinary system is incompatible with the patient’s life. Unilateral pathologies abnormal development kidney:

Right-sided agenesis. It makes up a small proportion of all pathologies and represents an anomaly in the structure of the right kidney.
Agenesis of the left kidney. Occurs rarely, no more than 7% of all clinical cases. Like the right-sided one, it can occur with or without preservation of the ureter.

These varieties birth defects little different from each other. In both cases, the remaining kidney hypertrophies, increases in size and adapts to dual function.

Agenesis of the right kidney may be misdiagnosed as aplasia. This is due to the fact that the ureter may be present and even function successfully. At the same time this pathology occurs more often, this is associated with anatomical location right kidney. The organ is located lower, it is more mobile and slightly smaller in size.

Signs of abnormal development

The absence of one of the organs affects the work of the other with increased load. Because of this, agenesis may often not be noticed in a newborn, and the anomaly will be detected only after some time with the help of diagnostic measures(computed tomography, ultrasound examination or urography).

The child may exhibit the following external signs:

some puffiness skin faces;
slightly flat nose - the bridge of the nose is wider than usual, together with a flat back;
the lobes of the frontal zone are quite convex;
ears located below normal indicators or with deformed areas.

Agenesis on the left side has more pronounced symptoms:

the volume of urine per day is significantly increased;
frequent urge to urinate;
feeling of nausea, possibly vomiting;
pain syndrome in the groin area.

In males, agenesis is more common due to the structural features genitourinary system. Specific symptoms are expressed due to the absence of a duct, which is responsible for the removal of seminal fluid and is caused by pain in groin area with ejaculation, the sacral region, sexual dysfunction (impotence) and infertility are possible.

In most cases, female agenesis is accompanied by various pathologies uterus, possible underdevelopment of the vagina or organ hypoplasia.

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Treatment and prevention

If the only healthy kidney functions normally, there are no symptoms and a characteristic enlargement of the organ is observed, then doctors will not use any additional actions in order to improve the functioning of the urinary system. The nephrologist will only prescribe dynamic observation monitor the kidney's ability to produce and excrete urine, regular urine tests and ultrasound examinations.

Also provided preventive actions, which are aimed at preserving the functionality of the kidney:

a diet that helps reduce the load on the only healthy organ is prescribed by a doctor, taking into account the individual characteristics of the patient;
restrictions related to contact types sports (boxing, wrestling) to protect a healthy organ from injury and unnecessary damage;
try not to lift heavy objects;
maintain a constant body weight, avoid sudden weight loss;
give up alcohol abuse and nicotine addiction;
support the immune system with vitamins and minerals;
avoid hypothermia to avoid the occurrence of infectious processes.

In the absence of function of the second kidney or with severe development of agenesis, when a healthy organ cannot cope with its work, intoxication occurs with decay products and toxic substances. IN in this case hemodialysis and kidney transplantation are prescribed.

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The term “renal aplasia” refers to a developmental abnormality in which one or both kidneys are absent or incompletely developed. A synonym for this condition is “agenesis”.

The disease is quite common; the anlage of one organ occurs in 1 in 1000 births and is often an accidental discovery already in adulthood.

Bilateral renal aplasia is very rare; it is combined with multiple anomalies of other organs that are incompatible with life. Right-sided aplasia is considered more favorable than left-sided aplasia.

Bilateral renal aplasia with absence of ureters, malformations of the genital organs, lung tissue. The pathology is incompatible with life.
Unilateral aplasia with the absence of the ureter and malformations of the genital organs occurs in 70% of girls.
Unilateral aplasia with the presence of a ureter and without anomalies in the development of the genital organs.

Causes of the anomaly

The cause of kidney agenesis is the absence or arrest of the development of the Wolffian duct in half of the urinary tract; the metanephric duct does not develop to the metanephrogenic blastema. TO this state The following factors may lead to:

Transferred acute respiratory diseases in the first trimester of pregnancy.
Hormonal diseases in the mother - diabetes mellitus, Cushing's disease or syndrome.
Preservation of pregnancy hormonal drugs, which contain synthetic progestins.
Increased ionizing radiation.
Sexually transmitted diseases – ureplasmosis, chlamydia.
Chronic alcoholism and tobacco smoking.

Clinical picture

The clinical picture of this pathology depends on the type of anomaly. With bilateral organ agenesis, fetal death occurs in the last trimester of pregnancy, or the newborn dies in the first week of life from the development of acute renal and cardiovascular failure.

With unilateral aplasia with unchanged ureters and genitals, symptoms may be completely absent. Often this pathology is found during routine or preventive ultrasound examination. One kidney copes with its function sufficiently in the absence of chronic infectious diseases other organs and systems.

When a bacterial or infectious agent enters, glomerulonephritis or pyelonephritis develops, the load on the organ increases and renal failure develops, and the following symptoms occur:

Swelling of the limbs and puffiness of the face.
Increased blood pressure.
Constant dull pain in the lower back or groin area.
Renal colic from an underdeveloped kidney due to the proliferation of fibrous tissue.
Severe intoxication syndrome - increased body temperature to 39 degrees, headache, weakness, lack of appetite.
Vomiting, diarrhea.

Unilateral aplasia with underdevelopment of the ureters and genital organs is diagnosed in the first days of a newborn’s life. Clinically manifested by oliguria (decreased daily amount urine), swelling of the face and lower limbs, constant pain in the area of the ureter, which is manifested by restlessness and crying of the child. In case of incorrect diagnosis and further treatment chronic renal failure develops.

The gold standard for diagnosing renal agenesis is X-ray examination , including excretory urography, angiography of the renal vessels and cystoscopy. Radioisotope and ultrasound examinations are less informative and more expensive.

On excretory urography no contrast appears on the aplasia side, indicating the absence of renal vessels.

Cystoscopy reveals no ureteral orifice and reveals unilateral atrophy of the vesical triangle.

Ultrasound examination and vascular angiography show no signs of blood circulation and the ureter is underdeveloped or absent. The pedicle and pelvis are absent or incorrectly formed. The contralateral kidney is hypertrophied due to a compensatory mechanism.

At laboratory methods research such as general and biochemical analysis blood and urine, it is possible to suspect kidney aplasia only at the stage of formation chronic failure organ.

On palpation, the only kidney is enlarged in size and rarely painful.

Treatment of kidney aplasia

For bilateral aplasia it is required immediate treatment, consisting of continuous hemodialysis and further transplantation of both kidneys.

With unilateral aplasia, the patient undergoes examination every six months with a mandatory assessment of urine excretion. A lifelong restricted diet is recommended. table salt and squirrel. Daily requirement in liquid - no more than 1.5 l.

Treatment according to syndromes:

Antihypertensive therapy – ACE inhibitors(enalapril, lisinopril), B-blockers (anaprilin, bisoprolol, metoprolol), sartans (Lozap, Lorista).
Diuretics– furosemide, veroshpiron, triamterene.
Antibacterial drugs if infection occurs - cephalosporins (ceftriaxone, ceficom), uroseptics, protected aminopenicillins (Amoxiclav, Augmentin).
Drugs that improve blood circulation– trental, pentoxifylline, chimes, dipyridamole.
General restorative therapy– vitamins of group B, C, E, A.

With the development of chronic renal failure, hemodialysis and surgical treatment in the form of organ transplantation.

Consequences and complications

In most cases, the prognosis for unilateral renal aplasia is favorable, but subject to diet and management healthy image life.

Prevention

Preventive measures aimed at avoiding renal aplasia should be carried out when planning pregnancy, include the sanitation of all existing foci of infection, cessation of smoking and drinking alcohol, strengthening the general resistance of the body, timely vaccination, treatment of the sexual partner of all chronic diseases, avoiding prolonged exposure to ionizing radiation.

To understand what renal aplasia is, it is necessary to understand the concept of agenesis, since this is one of its forms.

Agenesis itself - pathological condition, in which there is a complete absence of a kidney or its leg, which is a congenital defect.

Aplasia - failure intrauterine development, with preservation of the ureter and blood vessels, but the kidney itself is poorly developed, looks like a rudiment, and cannot function normally.

With pathology, all functioning falls on the other kidney, resulting in an increased load.

Main reasons

According to statistics, kidney aplasia is diagnosed in 1 person out of 1200 other people.

A similar anomaly appears due to insufficient development of the metanephros duct.

The defect is accompanied by a normal or short ureter, and in some cases the child will not have one at all.

Agenesis and aplasia of the kidney are considered birth defects in development. Often, children with similar anomalies experience death, death can occur in the womb or immediately after birth.

In situations where the second kidney can function normally and compensates for the deficiency of the second part of the paired organ, the disease can be diagnosed at an older age during a routine examination.

In some cases, the main symptoms of the pathology do not appear, their development is slow, therefore anomalous structure can only be determined in old age.

Renal aplasia often causes kidney diseases, including:

Increased intrarenal pressure.
Pyelonephritis different types and shapes.
Urolithiasis.

In medicine, there are several types of defects, and the least likely person will have a lack of kidneys.

This condition is the most dangerous; a child cannot live without a paired organ, so death occurs immediately after birth. Much more often, newborns do not have one kidney on either side.

Knowing what it is, it is important to understand possible reasons formation of a congenital defect.

Doctors and scientists cannot say for sure why children are born with only one kidney, but it should be noted that genetics and hereditary predisposition do not take part in the anomaly. Due to this, the defect is considered congenital.

The formation of a paired organ occurs from the 5th week of pregnancy. This process continues throughout development in the womb, making it difficult to determine the exact causes.

Kidney aplasia can be caused by several factors:

Infection with viruses and other infections during pregnancy, namely in the 1st trimester. As a rule, the danger is rubella, influenza and similar diseases.
Conducting ionizing radiation during examination during pregnancy.
The presence of diabetes in a pregnant woman is also a possible cause.
The result of using powerful medications, hormonal medications. The problem arises not just from pills, but from their incorrect use or independent selection of drugs for the treatment of various diseases and disorders.
Abuse of alcohol or smoking during pregnancy and before conceiving a child, when no preparation is carried out.
Any types of sexually transmitted pathologies in a woman.
Usage contraceptive drugs before the start of pregnancy.
Any disruption of the endocrine system.

The described reasons must be taken into account by doctors during pregnancy.

Types of violations

Used in medicine special classification, which makes it possible to determine the form of the violation:

Bilateral deviation – absence of a paired organ. This type of defect is the most dangerous, is diagnosed extremely rarely and causes the death of the fetus after birth or in the womb.
Agenesis of the right or left kidney is a condition in which the child does not have one part of the paired organ with the left or right side. In this case, one kidney takes over the main functions and loads.
Aplasia of the right or left kidney is characterized by incomplete formation of the kidney on one side. In this case, the part will look like a rudiment, which does not have basic structures, and therefore cannot work.

Quite often, agenesis of the right kidney is diagnosed and predominantly females suffer from the anomaly.

Doctors hypothesize that the peculiarity lies in the greater mobility of the right kidney, as well as its smaller size.

The defect can often be identified in the first months of a child’s life. In this case, the following symptoms develop:

The child spits up frequently.
Increased urine production.
Severe dehydration.
The appearance of high blood pressure and attacks of hypertension.
Rapid development of paired organ failure.

It is worth noting that the defect on the right may go unnoticed, but as a result of diagnosis and examination internal organs, the anomaly is determined randomly.

If we talk about right-sided kidney aplasia, the signs are almost invisible. This condition has a more favorable prognosis for the child’s life.

Provided that a healthy kidney can fully compensate for the deficiency of the paired organ. In rare situations, with right-sided aplasia, nephropathy appears in children, high blood pressure and other diseases.

Agenesis of the left side occurs with more severe symptoms and consequences, since the main load falls on the right side.

Physiologically, the right kidney differs in size and mobility from the left. With such an anomaly, patients develop the following types of complications:

Frequent pain in the groin area.
Problems with ejaculation in adulthood.
Disturbances in sexual functions.
Infertility.
Impotence.

Aplasia of the left kidney is determined very rarely in medicine, only in 7% of all cases.

Quite often, this problem is supplemented poor development other internal organs.

Left-sided aplasia is typical for men and boys, complemented by the absence of the vas deferens, as well as impaired development of the bladder.

If the defect appears in girls or is diagnosed in women, then there will be additionally incomplete development of the uterine appendages, ureters, and uterine septa.

IN healthy organ with an anomaly, a cyst often appears, but the tissue structure is not severely affected, and therefore is within normal limits.

Main symptoms

Kidney aplasia always differs from agenesis in less pronounced signs and the strength of their manifestations. Often the defect passes without characteristic manifestations.

Compared to a right-sided or left-sided problem, the symptoms will be stronger if the left kidney is affected. The main ones are:

An increase in the volume of urine released throughout the day.
Frequent urge to urinate.
Attacks of vomiting.
An increase in blood pressure that cannot be controlled with special medications.
The appearance of folds on the body.
Development of pain that radiates to the lumbar region and sacrum.
The appearance of sexual dysfunction in men.
General signs of deteriorating health and well-being.
Swelling on the face.

To determine pathological formation V childhood, parents should monitor for the following symptoms:

Puffiness of the face.
A wide nose that has a flattened back.
Low position of the ears.
Large belly size, which is not typical for children.
The appearance of folds on the skin.
The placement of the eyes is excessively wide.

The described visual features indicate an anomalous structure, but this can only be confirmed after a comprehensive diagnosis.

The described condition does not always indicate a defect. In children with severe course anomalies, signs of severe intoxication appear as a result metabolic processes, failure of the paired organ develops.

Diagnosis

To determine the defect, you will need to collect basic data as a result visual inspection, as well as collecting complaints and symptoms.

The doctor writes down the basic information that the patient or his parents say, as well as find out the first symptoms and the factors that provoked them.

In addition, doctors need to be aware of other abnormalities that may occur in children or adults.

Kidney aplasia should be properly distinguished by doctors from other disorders or the usual inability to function in a paired organ.

To establish an accurate diagnosis, aortography is performed; with its help, a non-functioning kidney is distinguished from aplasia. An anomaly can be determined using the following methods:

Angiography.
Ultrasound and x-rays are used to determine the condition of the healthy part of the paired organ.

To obtain information about overall health, blood and urine tests are used, as well as urine cultures.

Treatment

Treatment of the defect can only be carried out for certain indications and the person’s condition:

There are frequent and severe pain in the back, lower back, and kidney area.
Nephrogenic hypertension appears.
Reflux occurs into the urinary canal, which is not fully developed.

Often with right-sided aplasia special holding no therapy required. Patients simply need to use proper nutrition, which allows you to reduce the load on the healthy part of the paired organ.

The diet consists of giving up:

Fried.
Spicy.
Fat.
Smoked.
Salty.

Patients should not use sauces, especially hot ones, herbs and spices, as well as sour juices and fruits.

The use of soda, alcohol, coffee and tea is strictly prohibited. At pronounced signs For abnormalities, medications may be prescribed, including:

Painkillers.
Diuretics.
Medicines to eliminate tissue swelling.

Left-sided aplasia also does not require special treatment; therapy is prescribed in rare cases.

To support health and normal operation A healthy kidney requires the use of prevention rules, which do not allow infectious diseases to develop.

By increasing and controlling the state of the immune system, with one functioning kidney, people are able to live normally.

Particular attention should be paid to children who have bilateral aplasia or agenesis.

Just a few years ago, when similar condition the child was simply dying; today medicine has the ability to save life through kidney transplantation in newborns.

The procedure is difficult and requires a quick response to select a donor. Through surgery, it is possible to restore and maintain a normal life.

In the most severe cases, a special commission assigns disability. Surgical treatment can be carried out at very intense pain from the abnormal side, as well as the development of complications. In this case, the pathological kidney is removed along with the ureter.

Prevention

In order for a child to be born without anomalies, any woman needs to prepare for pregnancy, use the rules of prevention before conception and during gestation. The basic rules are:

Completely give up alcohol and smoking.
Plan a pregnancy.
Conduct timely treatment all diseases, especially those of an infectious nature.
Use medications only as prescribed by a doctor.
Control your diet and use only the right, balanced diet.
It is normal to react to stressful situations.

If a child is not immediately diagnosed with a defect, then in the future, when it is identified, it will be necessary to immediately change their lifestyle, adhering to healthy principles, adjust your diet.

If pain and other signs of anomaly develop, then self-therapy is excluded. You need to see a doctor who will give you basic recommendations.